Marine Boudot de la Motte1*, Thierry Maisonobe1, Charline Benoit2, Yann Nadjar2, Nathalie Vernier3, Véronique Leblond4, Jean-Michel Vallat5 and Karine Viala1
1Department of Clinical Neurophysiology, CHU Pitié-Salpêtriere, France
2Department of Neurology, CHU Pitié-Salpêtriere, France
3Department of Internal Medicine, Private Hospitals of Metz, France
4Department of Hematology, CHU Pitié-Salpêtriere, France
5Department and Laboratory of Neurology, CHRU Limoges, France
Background and Aims: Waldenström's Macroglobulinemia (WM) associated neuropathy is frequent. However, it can have different etiologies leading to different treatments, which makes the diagnosis challenging for the clinician. Methods: Case study. Results: We report the case of a 71-year-old female who presented a severe painful sensory motor axonal neuropathy. She had an IgM lambda monoclonal gammopathy with bone marrow and pleura lymphoplasmocytic cell infiltration, in favor of WM. The neuropathy worsened despite two lines of hematologic treatment. Nerve biopsy showed diffuse infiltrates of B and T cells with a B cell amplification confirmed on polymerase chain reaction based clonality testing. Interpretation: Nerve biopsy is a key tool in the diagnosis of WM associated axonal neuropathy, especially for tumoral nerve infiltration since other classical investigations are often negative. The pathogenesis of this infiltration is debated, but our data are in favor of a hematogenic invasion by tumoral cells. Neuropathy secondary to tumoral nerve invasion may worsen in spite of good hematological response to treatment.
EMG; Hematologic; Peripheral neuropathy; Nerve tumor; Biopsy
de la Motte MB, Maisonobe T, Benoit C, Nadjar Y, Vernier N, Leblond V, et al. Severe Worsening Axonal Neuropathy in Waldenstrom's Macroglobulinemia: Benefit of Nerve Biopsy. Ann Clin Case Rep. 2021; 6: 2024..